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Ann Thorac Surg 2001;72:2077-2080
© 2001 The Society of Thoracic Surgeons
a Departments of Pediatrics and Surgery, Children’s Hospital and Regional Medical Center, and the University of Washington School of Medicine, Seattle, Washington, USA
b Division of Cardiology, Children’s Hospital and Regional Medical Center, and the University of Washington School of Medicine, Seattle, Washington, USA
c Division of Cardiac Surgery, Children’s Hospital and Regional Medical Center, and the University of Washington School of Medicine, Seattle, Washington, USA
Accepted for publication August 17, 2001.
* Address reprint requests to Dr Duncan, The Cleveland Clinic Foundation, Pediatric and Congenital Heart Surgery/M41, 9500 Euclid Ave, Cleveland, OH 44195, USA
e-mail: duncanb{at}ccf.org
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Abstract |
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Methods. Our institution has offered first-stage palliation for all such patients regardless of age. The results of this policy were reviewed.
Results. Nine patients older than 30 days (median age 67 days, range 36 to 108 days) with diagnoses of hypoplastic left heart syndrome (n = 5), double-outlet right ventricle with hypoplastic aortic arch (n = 2), unbalanced atrioventricular septal defect (n = 1), or single left ventricle with subaortic stenosis (n = 1) underwent surgical palliation. Patients underwent a Norwood (n = 7) or Damus-Kaye-Stancel (n = 2) procedure with a 4- or 5-mm modified Blalock-Taussig shunt; all patients survived the operation. Eight patients underwent a subsequent bidirectional Glenn (2 perioperative deaths, both due to pneumonia; 6 survivors). Two of the 6 surviving patients have undergone Fontan reconstruction and 4 are awaiting Fontan.
Conclusions. Surgical palliation for complex univentricular cardiac malformations can be performed in older infants with results comparable to those in neonates. The use of a larger shunt may contribute to these improved outcomes.
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Introduction |
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We performed initial palliative surgical procedures in 9 children older than 30 days of age who had anatomy typical of hypoplastic left heart syndrome or its variants, with all of these children surviving to hospital discharge. We undertook the present study to evaluate these patients to determine which factors contributed to this successful approach.
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Material and methods |
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Operative technique
Patients underwent a Norwood procedure (7 patients) or Damus-Kaye-Stancel and shunt (DKS-shunt) procedure (2 patients). Briefly, for the Norwood procedure, the operative technique included arterial cannulation of the proximal pulmonary artery with venous drainage through a single cannula placed in the right atrium. The procedure was performed with deep hypothermic circulatory arrest after a single dose of blood containing cardioplegia. An atrial septectomy was performed in each patient. The pulmonary artery was anastomosed to the ascending aorta and arch augmentation was performed with pulmonary homograft. For the DKS-shunt procedure, the distal ascending aorta was cannulated directly and no period of circulatory arrest was required. The ascending aorta was anastomosed to the proximal pulmonary artery with augmentation of the anterior aspect of the anastomosis by pericardium as described elsewhere [3].
Our standard approach for newborns undergoing first-stage palliation is to use a 3.5-mm Gore-Tex interposition graft (W.L. Gore and Assoc, Flagstaff, AZ) between the innominate artery and the central pulmonary artery for the modified Blalock-Taussig shunt. In these 9 older patients we used a 4-mm (3 patients) or a 5-mm (6 patients) shunt that was inserted after the reinstitution of cardiopulmonary bypass during systemic warming. The larger shunt size was selected to overcome higher pulmonary vascular resistance that might exist in these patients presenting late for operation.
Six of the 8 patients undergoing bidirectional Glenn shunt had anastomosis of a right superior vena cava to the right pulmonary artery using cardiopulmonary bypass without aortic cross-clamping. One patient with bilateral superior vena cavas underwent a bilateral, bidirectional Glenn shunt. One patient required simultaneous central pulmonary artery patch reconstruction with autologous pericardium to treat moderate central and left pulmonary arterial stenosis. The remaining 7 patients were deemed to have adequate pulmonary artery architecture for a successful bidirectional Glenn shunt.
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Results |
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Comment |
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TopAbstractIntroductionMaterial and methodsResultsCommentReferences |
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Poorer outcomes for these children have been mainly attributed to elevated pulmonary vascular resistance that these children possess after an extended period of excessive pulmonary blood flow. Elevated pulmonary vascular resistance presumably makes postoperative management more complicated because of difficulties in achieving the appropriate balance between the pulmonary and systemic circulations. Hypoxemia and sudden fluctuations in oxygenation due to the reactive nature of the pulmonary vasculature that exists in these children might be expected during their postoperative course. Sudden and severe increases in pulmonary vascular resistance might also be expected that could lead to fatal pulmonary hypertensive crises. In addition, chronic volume loading of the single ventricle has been implicated as a possible etiology for perioperative ventricular failure after first-stage palliation in these patients [1, 2].
The present series agrees with other reports that have not found advanced age to be a risk factor for first-stage palliation of hypoplastic left heart syndrome and its variants [5–9]. These reports have in general evaluated only a small number of older infants as part of a large series of infants undergoing first-stage palliation. The present study focused specifically on the outcomes for 9 consecutive, older infants after first-stage palliation. Our findings are in agreement with an earlier study that also focused on this patient group and demonstrated good results with the Norwood procedure in these older infants [10]. Rossi and colleagues [10] described 20 infants with hypoplastic left heart syndrome, many of whom had originally been listed for transplantation who eventually underwent a Norwood procedure beyond 2 weeks of age. Eighteen of the 20 patients (90%) survived in this experience with survival in all 4 infants who underwent operation at more than 30 days of age.
It has been our experience that modifying the operative procedure with placement of a relatively large Blalock-Taussig shunt, along with the usual precautions to balance the systemic and pulmonary circulations during postoperative care, mitigates the risk for these patients. The use of a 4- or 5-mm modified Blalock-Taussig shunt is critical to this successful approach. This larger caliber shunt originating from the innominate artery with implantation into the central pulmonary artery provides pulmonary blood flow adequate to overcome higher pulmonary vascular resistance. This source of increased pulmonary blood flow allows standard postoperative management to be successful in these patients. Although in our postoperative management we sought to avoid factors that might exacerbate increased pulmonary vascular resistance, such as respiratory acidosis, no extraordinary measures were required to safely recover these patients. Similarly, none of these patients demonstrated pulmonary overcirculation with systemic hypoperfusion in the immediate postoperative period. A 4- to 5-mm modified Blalock-Taussig shunt appears to provide a good "physiologic fit" in terms of providing an appropriate balance between systemic and pulmonary circulations. Indeed, none of these patients were particularly difficult to manage in the postoperative period; all had minimally complicated postoperative courses and no patient had documented pulmonary hypertensive crises.
Mortality in this group was much higher than expected at the time of the bidirectional Glenn shunt (2 of 8 patients, 25%) with both nonsurvivors succumbing to pneumonia. Neither of these patients was considered to be at increased risk for second-stage palliation nor had either patient demonstrated pulmonary infections before the bidirectional Glenn shunt. It is possible that late presentation for first-stage palliation predisposes these patients to problems with subsequent steps in the palliative sequence. Perhaps prolonged periods of increased pulmonary blood flow before first-stage palliation with resultant elevations in pulmonary vascular resistance and the need for larger shunts made these patients more susceptible to pulmonary infection. Further, it is possible that the pneumonias seen after the bidirectional Glenn shunt were sequelae arising from more significant underlying problems of poor hemodynamics related to elevated pulmonary vascular resistance.
Alternative strategies exist to successfully provide long-term palliation for this patient group. If larger shunts contribute to poor outcomes after the bidirectional Glenn shunt, these patients could be managed with standard sized shunts with anticipation and treatment of perioperative elevations of pulmonary vascular resistance. Aggressive use of nitric oxide and even mechanical circulatory support could be used to manage these patients after first-stage palliation with smaller shunts. It might be possible to achieve more acceptable hemodynamics and pulmonary artery pressures in the long-term with these alternative approaches. Alternatively, waiting for a longer period than the 6 months between first- and second-stage palliation used in this series may result in further reductions of pulmonary vascular resistance.
With the management points described above in mind, we conclude that presentation at older than 30 days of age does not confer excessive risk for the performance of first-stage palliation. Based on these data, the policy of offering first-stage palliation for infants regardless of lateness of presentation would appear to be justified. Perhaps most importantly, this series provides additional information that can be considered during the preoperative discussion of risk with parents of children who present late for first-stage palliation.
Infants with hypoplastic left heart syndrome and its variants who present at older than 30 days of age for first-stage palliation can be treated successfully. The placement of a 4- or 5-mm modified Blalock-Taussig shunt at the time of the surgical procedure overcomes elevated pulmonary vascular resistance in these children and allows standard postoperative management to be successful in achieving a balanced circulation.
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